Gliederung

Objective: Patients suffering from von Hippel-Lindau disease (VHL), a rare autosomal-dominantly inherited disorder, often develop multiple spinal intramedullary hemangioblastomas. Indications for surgical treatment of spinal hemangioblastomas in VHL patients are still controversial, especially when tumors are detected by magnetic resonance imaging in asymptomatic patients. For this study, we reviewed a series of VHL patients operated for spinal hemangioblastomas to evaluate indications, surgical strategies and outcome.

Methods: This series consists of a total of 41 VHL patients operated at our department between 1993-2006. All tumors were diagnosed by magnetic resonance imaging. All but one patient had multiple tumors. Surgery was indicated if tumor or pseudocyst growth occured, regardless of accompanying symptoms.

Results: Surgical approach to the spine was performed by partial hemilaminectomy, hemilaminectomy or laminectomy of one or more segments, depending on tumor presentation. All tumors were removed completely. Mean operating time was 4h (1.5-7.5h). Functional grades, according to the McCormick scale, were determined before and after surgery and at follow-up assessments. After the operation, 26% of the patients had improved functional grades and 77% of the patients remained neurologically stable. 10 asymptomatic patients were treated for progressive tumor or pseudocyst growth on serial MRI scans. All of these patients remained asymptomatic after the operation. Three patients with very extensive confluencing pseudocysts and multiple hemangioblastomas were worse postoperatively, 2 of these required further operations for progressive worsening of symptoms and 1 temporarily went from McCormick grade II to III.

Conclusions: In the presence of multiple, often confluencing lesions with large pseudocystic components, the difficulty is to determine the lesion most likely to cause the patient's symptoms and to weigh the benefits of microneurosurgical treatment against potential post-surgical neurological deterioration. Intramedullary hemangioblastomas in VHL patients can be surgically removed with low morbidity-rates and excellent clinical outcome in the majority of cases. Treatment should be considered for asymptomatic lesions in patients with good neurologic function before neurologic deterioration occurs. Surgical treatment of clinically stable patients (symptomatic or asymptomatic) with very large or multiple confluencing cystic tumors should be indicated only after careful evaluation, since neurologic deterioration is more likely to occur in these cases.